by Meredith Rainey in CultureMap Austin
published February 6, 2012
Our job as parents is to protect our children. So what do you do when you find out you unknowingly passed a rare and incurable genetic disease on to your only daughter? If you are Charlotte and Craig Benson, you catch your breath, lean on your faith and after the initial shock fades some, you choose to tackle the greatest challenge of your lives head on — you form a foundation to find a cure to try to save your daughter’s life, and the lives of hundreds of other children confronting a fate no child should ever have to face.
The Bensons, who founded the Beyond Batten Disease Foundation in 2008 after their then 5-year-old daughter Christiane’s Batten Disease diagnosis, have a lot they’d like to accomplish this year, including launching a screening test for Batten Disease and hundreds of other rare genetic diseases with devastating effects.
Their foundation has enlisted the talents of country music superstar Keith Urban to help raise money for and awareness of the cause they hold so dear to their hearts. Just in time for Valentine’s Day, Urban is headlining the organization’s annual fundraiser on Feb. 10 at ACL Live at The Moody Theater.
“Date Night with Keith Urban” features a VIP party, a live auction and of course a stellar concert crafted by an artist whose long list of awards includes being named CMA Entertainer of the Year.
The money raised from the event will help launch the genetic screening test the foundation helped pioneer. It will also go towards the Benson’s ultimate goal of finding a cure for the fatal neurodegenerative disease that’s taking their daughter’s sight and currently has no known treatment.
“Hopefully over the next generation we’ll see a reduction in incidence of all of these [diseases] and I think that would be a great legacy for us and for Christiane,” Craig Benson says.
Charlotte Benson says Christiane was a beautiful baby, complete with ten fingers and ten toes. The proud parents describe the day she was born as the happiest day of their lives. They never would have imagined this perfect little girl may not live to graduate high school or have a family of her own.
“She had a perfectly normal birth and we had every reason to believe that she would be a perfectly normal child. And then, when she got into kindergarten, when she started to read, she put books really close to her face and so we thought she just needed some glasses. So we took her to the eye doctor, and then one appointment led to another and we ended up in the neurologist’s office. Six weeks later, we had a diagnosis of Batten Disease,” explains Charlotte.
Batten disease is an inherited disorder of the nervous system that is fatal. Early symptoms can appear between age 5 and 10 and may start with vision problems and seizures. Children with Batten Disease eventually go blind and become physically and mentally incapacitated; they don’t live past their teens or early 20s.
That kind of news would make many parents want to crawl into bed and stay there. A few months after the devastating news, the Bensons made a decision: they weren’t going to let this disease define them — they were going to try to define it.
“There were a couple of evolutions of this. Obviously at first, we didn’t want to do anything. You know, the news kind of knocks you down for a bit. I think as we started sharing our story with close friends and family, I think that’s where we got some of the support and strength that we got initially from our faith… we started getting the grasp that there was maybe something we could do to help this,” Craig says.
Charlotte adds, “When we first started the foundation, it’s the absolute horror of a doctor telling you to go home and there’s nothing you can do about it except make your child comfortable and watch the story unfold. And so, as a parent, your instinct is to do something to try to change this and to make a difference not only for her but for so many other kids who are stricken with the same disease.”
The Benson’s say they soon realized they wouldn’t be alone in their mission.
“We found ourselves surrounded with an incredibly golden circle of friends with enormous talent, intelligence, resources and contacts. We’re in a really unique position not only with the friends and community surrounding us. That coupled with Craig’s position as CEO of Rules Based Medicine and all of his contacts to every brilliant medical doctor and scientist, those two things combined made it a natural fit to try to go forward and do something to make a difference,” Charlotte says.
With the support of their family, friends and neighbors and Craig’s connections in the biomedical industry, the Bensons had what they needed to start their mission to find a cure for their Christiane.
They named their foundation the Beyond Batten Disease Foundation, not knowing at the time how appropriate that title would be. Work funded by the foundation has resulted in the creation of a test to screen for not only Batten Disease, but also for approximately 600 other rare genetic diseases. Though the diseases may be rare themselves, collectively they affect thousands of children.
With a simple cheek swab, couples can be screened to find out if they are both carriers for a long list of genetic diseases they may pass on if they have children.
“Part of our mission has certainly resonated with everyone because it does affect everybody,” Craig says. “These rare genetic mutations that obviously affect us — everyone has some. We knew it was very critical early on for us to reach an audience outside of this small group of 500 families in the US that are directly affected [by Batten Disease].”
He says an added benefit is that royalties from the screening test will provide a revenue stream to fund the foundation’s other projects. The Benson’s say while the screening test, which is currently undergoing beta testing before being launched nationally, is a great start, their ultimate goal is to help eradicate Batten Disease — to find a cure for their little girl.
Christiane is now nine years old and in the third grade. Her parents are trying to keep life for her and her younger brother Garland as normal as possible, while managing her symptoms and taking things the only way they can — as they come.
“[Christiane] knows she has Batten disease. She knows that she’s going to be blind and we told her that that there are some other things that come with it, but we’re sort of crossing that bridge as her symptoms reveal themselves,” Charlotte says.
The Bensons cherish every day and every moment they have with Christiane, while trying to stay hopeful. Charlotte says the horrible diagnosis they got four years ago changed their perspective and their paths forever.
“It’s strengthened our faith. We’ve seen the goodness in people that we’ve never known before, and seen a community of people surround us and empower us on our mission. You never take a single moment for granted with your children – you live for today.”
If you’d like to be part of the biggest fundraiser of the year for the Beyond Batten Disease Foundation and want to see what makes Keith Urban such a sought after entertainer, you’ve got your chance on Feb. 10 at The Moody Theater.
http://austin.culturemap.com/newsdetail/02-06-12-21-38-keith-urban-lends-talents-to-raise-money-for-austin-based-foundation-aimed-at-curing-rare-genetic-disease-affecti/
